Hemolytic uremic syndrome

Year 2011, , 519 - 525, 01.12.2011

Faruk Öktem Ayça Esra Kuybulu

https://doi.org/10.5798/diclemedj.0921.2011.04.0081

Abstract

Haemolytic uremic syndrome (HUS) is a severe disease with microangiopathic anemia, thrombocytopenia and leading cause of acute renal failure in children. Several etiological factors causing to HUS have been identified, like infections, genetic mutations, drugs, systemic dis­eases. In this review, we present the new classification of the disease, detailed information about pathogenesis, diagnostic methods and therapeutic approaches.

Keywords

Hemolytic uremic syndrome, child, new ap­proaches

Hemolitik üremik sendrom

Abstract

Hemolitik üremik sendrom (HÜS) mikroanjiyopatik anemi, trombositopeni ve çocuklarda en sık akut böbrek yetmez­liği nedenlerinden biri olan ciddi bir hastalıktır. Enfeksiyon­lar, genetik mutasyonlar, ilaçlar, sistemik hastalıklar gibi HÜS\'e sebep olan birçok mekanizma tanımlanmıştır. Bu derlemede, hastalığın yeni sınıflandırılmasını, patogene­zi, tanı yöntemlerini ve tedavi yaklaşımlarını sunacağız.

Keywords

Hemolitik üremik sendrom, çocuk, yeni yaklaşımlar.

References

• Besbas N, Karpman D, Landau D, et al. European Paediatric Re- search Group for HUS. A classification of hemolytic uremic syn- drome and thrombotic thrombocytopenic purpura and related dis- orders. Kidney Int 2006;70(3):423-31.
• Davis ID, Avner ED. Hemolityc uremic syndrome. In: Kliegman RM, Behrman RE, Jenson HB, et al. Nelson Textbook of Pediatrics, 18. edn. Saunders, An Imprint of Elsevier 2007:2181-3.
• Johnson S, Taylor CM. Hemolytic Uremic Syndrome. In: Avner ED, Harmon WE, Niaudet P, Yoshikawa N, eds. Pediatric Nephrology, 6th edn. Berlin Heidelberg: Springer-Verlag, 2009:1155-81.
• Palermo MS, Exeni RA, Fernández GC. Hemolytic uremic syn- drome: pathogenesis and update of interventions. Expert Rev Anti Infect Ther 2009;7(6):697-707.
• Scheiring J, Andreoli SP, Zimmerhackl LB. Treatment and outcome of Shiga-toxin-associated hemolytic uremic syndrome (HUS). Pe- diatr Nephrol 2008;23(10):1749-60.
• Blackall DP, Marques MB. Hemolytic uremic syndrome revisited: Shiga toxin, factor H, and fibrin generation. Am J Clin Pathol 2004;121(Suppl):81-8.
• Tarr PI. Shiga toxin-associated hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: distinct mechanisms of pathogenesis. Kidney Int Suppl 2009;112(1):29-32.
• Centers for Disease Control and Prevention (last update day: 26 July 2007) Hemolytic Uremic Syndrome, Post-diarrheal (HUS) http:// www.cdc.gov/ncphi/od/ai/casedef/ hemolyticcurrent.htm, son ulaşım tarihi: 23 Ağustos 2010
• Brandt J, Wong C, Mihm S, et al. Invasive pneumococcal disease and hemolytic uremic syndrome. Pediatrics 2002;110(2):371-6.
• Lee CF, Liu SC, Lue KH, Chen JP, Sheu JN. Pneumococcal pneu- monia with empyema and hemolytic uremic syndrome in children: report of three cases. J Microbiol Immunol Infect 2006;39(4):348- 52.
• Skerka C, Józsi M, Zipfel PF, Dragon-Durey MA, Fremeaux-Bac- chi V. Autoantibodies in haemolytic uraemic syndrome (HUS). Thromb Haemost 2009;101(2):227-32.
• Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. N Engl J Med 2009;361(17):1676-87.
• Fang CJ, Richards A, Liszewski MK, Kavanagh D, Atkinson JP. Advances in understanding of pathogenesis of aHUS and HELLP. Br J Haematol 2008;143(3):336-48.
• Loirat C, Noris M, Fremeaux-Bacchi V. Complement and the atypical hemolytic uremic syndrome in children. Pediatr Nephrol 2008;23(11):1957-72.
• Kavanagh D, Goodship TH. Update on evaluating complement in hemolytic uremic syndrome. Curr Opin Nephrol Hypertens 2007;16(6):565-71.
• Jokiranta TS, Zipfel PF, Fremeaux-Bacchi V, Taylor CM, Good- ship TJ, Noris M. Where next with atypical hemolytic uremic syn- drome? Mol Immunol. 2007;44(16):3889-900.
• Kavanagh D, Richards A, Fremeaux-Bacchi V, Noris M, Goodship T, Remuzzi G, Atkinson JP. Screening for complement system ab- normalities in patients with atypical hemolytic uremic syndrome. Clin J Am Soc Nephrol 2007;2(3):591-6.
• Berkel AI. Atipik hemolitik üremik sendrom: patogenez ve teda- vi ile ilgili son gelişmeler. Çocuk Sağlığı ve Hastalıkları Dergisi 2006;49(4):322-32.
• Furlan M. Deficient activity of von Willebrand factor-cleaving pro- tease in thrombotic thrombocytopenic purpura. Expert Rev Cardio- vasc Ther 2003;1(2):243-55.
• George JN. ADAMTS13, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome. Curr Hematol Rep 2005;4(3):167-9.
• Zimmerhackl LB, Besbas N, Jungraithmayr T, et al. European Study Group for Haemolytic Uraemic Syndromes and Related Disorders. Epidemiology, clinical presentation, and pathophysiology of atypi- cal and recurrent hemolytic uremic syndrome. Semin Thromb He- most 2006;32(2):113-20.
• Sharma AP, Greenberg CR, Prasad AN, Prasad C. Hemolytic ure- mic syndrome (HUS) secondary to cobalamin C (cblC) disorder. Pediatr Nephrol 2007;22(12):2097-103.
• Dlott JS, Danielson CF, Blue-Hnidy DE, McCarthy LJ. Drug- induced thrombotic thrombocytopenic purpura/hemolytic uremic syndrome: a concise review. Ther Apher Dial 2004;8(2):102-11.
• Wu CY, Su YT, Wang JS, Chiou YH. Childhood hermolytic uremic syndrome associated with systemic lupus erythematosus. Lupus 2007;16(12):1006-10.
• Gomes AM, Ventura A, Almeida C, et al. Hemolytic uremic syn- drome as a primary manifestation of acute human immunodefi- ciency virus infection. Clin Nephrol 2009;71(5):563-6.
• Lowe EJ, Werner EJ. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome in children and adolescents. Semin Thromb Hemost 2005;31:717-30.
• George JN. The thrombotic thrombocytopenic purpura and hemo- lytic uremic syndromes: overview of pathogenesis (Experience of The Oklahoma TTP-HUS Registry, 1989-2007). Kidney Int Suppl 2009;112:8-10.
• Kiessling SG, Bernard P. Hemolytic Uremic Syndrome. In: Kies- ling SG, Somers GJ. eds. Pediatric Nephrology in the ICU. Berlin Heidelberg: Springer-Verlag, 2009:219-30
• Ariceta G, Besbas N, Johnson S, et al. European Paediatric Study Group for HUS. Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol 2009;24:687-96.